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Introducción. La hipertensión arterial pulmonar (HAP) es una enfermedad que presenta un elevado índice de mortalidad en la población pediátrica. Para su diagnóstico, el gold standard es la prueba de reactividad vascular pulmonar (PRVP), debido a que permite medir la respuesta vasodilatadora del lecho vascular pulmonar frente a la administración de moléculas con acción terapéutica, como el óxido nítrico inhalado (iNO). Esta prueba al ser positiva se asocia a un mejor pronóstico. En la actualidad existe incertidumbre y falta de consenso sobre la indicación y administración de iNO durante la PRVP. Objetivo. Describir el uso reportado en la literatura sobre iNO en PRVP en sujetos pediátricos con HAP. Métodos. Revisión sistemática exploratoria sensible en bases de datos PubMed, Epistemonikos, Cochrane, Scopus, Lilacs y Scielo, que describen el uso de iNO durante la PRVP en sujetos pediátricos con HAP. Resultados. se identificaron 8.906 artículos, de los cuales se seleccionaron 5 para la revisión cualitativa. La PRVP se realizó durante el cateterismo cardiaco derecho (CCD) en sujetosentre 2 semanas y 18 años de edad. Los diagnósticos fueron HAP primaria, idiopática y asociada a patología cardiaca congénita, cardiomiopatía y enfermedad pulmonar. Esta prueba fue realizada en sujetos sólo con soporte de oxígeno o con sedación profunda en ventilación mecánica invasiva, con dosis variables de oxígeno (21 y 100%) e iNO (3 y 80 ppm), o asociado a otras moléculas como iloprostol®, dilitiazem, sildenafil y/o epoprostenol. La administración de iNO disminuyó presión de arteria pulmonar y la resistencia vascular pulmonar, con mantención de presión arterial sistémica y gasto cardiaco y sin complicaciones asociadas a su uso. Conclusiones. Existen escasos estudios sobre iNO en PRVP pediátrica y con calidad metodológica limitada. El iNO se utiliza como método diagnóstico de vaso reactividad en sujetos pediátricos con HAP asociada a cardiopatía congénita, primaria o secundaria. Los protocolos para su uso son variables con dosis entre 20 y 40 ppm, con o sin uso de oxigeno adicional, con tiempos poco precisos y sin consenso en equipos de administración.

Background. Pulmonary arterial hypertension (PAH) is a disease that has a high mortality rate among the pediatric population. For its diagnosis, the pulmonary vascular reactivity test (PVRT) is considered the "Gold Standard", because it allows to measure the vasodilator response of pulmonary vascular circulation with the administration of molecules with therapeutic action, such as inhaled nitric oxide (iNO). This test, when positive, is associated with a better prognosis of the disease. Currently, there's uncertainty and lack of consensus on the indication and administration of iNO during the PVRT. Objetives. to describe use of iNO in PVRT in pediatric users with PAH reported in the literature. Methods. Scoping review of studies published between 1992 and 2021 in PubMed, Epistemonikos, Cochrane, Scopus, Lilacs and Scielo databases, which describe the use of iNO during PVRT in pediatric users with PAH, in English and Spanish. Primary and secondary studies with a sensitive search strategy were considered. Results. 8,906 articles were identified, 40 were selected by title, 8 by full text, and 5 for final qualitative review. Of the total of articles selected, 3 were primary and 2 secondary studies. PVRT was performed during right heart catheterization (RHC) in a population between 2 weeks and 18 years old. Diagnoses were primary PAH, idiopathic PAH and PAH associated with congenital heart disease, cardiomyopathy and pulmonary disease. This test was carried out in subjects on spontaneous ventilation with oxygen support or with deep sedation in invasive mechanical ventilation, with variable oxygen doses between 21 and 100%, with exclusive use of iNO between 3 and 80 ppm, being more used between 20 and 40 ppm, or associated with other molecules such as iloprostol®, dilithiazim, sildenafil and / or epoprostenol. In all selected studies, administration of iNO decreased PAP (pulmonary artery pressure) and PVR (pulmonary vascular resistance), with maintenance of SBP (systemic arterial blood pressure) and cardiac output. The primary studies were made up of pre and post-test of serial or parallel interventions. The selected studies of iNO in PVRT did not report complications associated with its use. Conclusions. studies on iNO in pediatric PVRT are scarce in number of publications and methodological quality. iNO is used as a diagnostic method of vasoreactivity in pediatric users with PAH associated with congenital, primary, or secondary heart disease. The protocols for its use are variable with recommended doses between 20 and 40 ppm, with or without the use of additional O2, with imprecise times and without consensus in administration equipment. The response to PVRT serves as a guide for the treatment and prognosis of pediatric users with PAH.

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BACKGROUND: The upper limb approach utilizing transradial access for combined left and right heart catheterization (CLRHC) and ultrasound-guided antecubital venous access for isolated right heart catheterization (IRHC) are strategies that may reduce risks, especially in anticoagulated patients. combined left and right heart catheterization. OBJECTIVES: To assess safety and feasibility of upper limb approach for IRHC or CLRHC in anticoagulated versus non-anticoagulated patients. METHODS: Ninety-three patients who underwent IRHC or CLRHC with ultrasound-guided antecubital venous access and transradial arterial access were prospectively enrolled. The primary outcome was a composite of procedure failure and incidence of immediate vascular complications. RESULTS: Of the 93 patients, 44 (47%) were on anticoagulation and 49 (53%) were not. Mean age was 54 ± 17 and 53 ± 15 years, respectively. Atrial fibrillation (39% vs 15%) and chronic kidney disease (21% vs 6%) were more common in anticoagulated patients. The main indication for anticoagulation was deep vein thrombosis/pulmonary thromboembolism in 22 patients (50%). The primary outcome occurred in 4 (8%) patients in the non-anticoagulated group as compared with 0 in the anticoagulated group (p = 0.12). Procedure failure occurred in two patients (4%) and immediate vascular complications in two patients (4%) in the non-anticoagulated group (p = 0.3 for all). There was no difference between groups regarding duration of the procedure, radiation dose, fluoroscopy time, post-procedure recovery room time and median time to venous or arterial hemostasis. CONCLUSIONS: The upper limb approach for heart catheterization had similar rates of procedure failure and immediate vascular complications in anticoagulated patients when compared to non-anticoagulated patients.

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RESUMEN: Antecedentes: La presión media arterial pulmonar (PMAP) es una variable hemodinámica indispensable para el diagnóstico, clasificación y pronóstico de la Hipertensión Pulmonar (HP). Su cuantificación se realiza en forma invasiva por cateterismo cardíaco derecho (CCD) y no invasivamente por ecocardiografía Doppler. Masuyama propuso su medición mediante el gradiente transvalvular pulmonar diastólico derivado de la velocidad máxima inicial de la regurgitación pulmonar (∆RPi2) correspondiendo cercanamente a la medición invasiva. Objetivos: Revalidar 3 métodos ecocardiográficos que estiman la PMAP y valorar la utilidad del método de Chemla en el Test de Reactividad Vascular Pulmonar (TRVP). Métodos: Estudio prospectivo, observacional, doble ciego divido en dos etapas. A) o I) 30 pacientes se realizó ecocardiografía Doppler diagnóstica en nuestro centro. Se midieron regurgitación tricuspídea (RT) y tiempo de aceleración pulmonar (TAP) para derivar las siguientes ecuaciones: 1) 0.61xPSAP+1.95 (Chemla) 2) Gradiente presión media RT (∆PmRT) +PAD (presión-aurícula derecha) (Aduen) y 3) 79-0.45xTAP o 90-0.60xTAP, según sea el valor del TAP. B) o II) 10 pacientes enrolados para realizar el TRVP comparando la medición ecocardiográfica (Chemla) con CCD. Resultados: En la primera parte del estudio se encontró alta correlación entre las 3 ecuaciones: ChemlaAduen, R2=0.91; Chemla-Kitabatake, R2=0.87; Aduen-Kitabatake, R2=0,91. En la segunda parte comparando la PMAP-Chemla y Cateterismo derecho (CD) obtuvimos alta correlación: en tiempo 0, 30 min y recuperación:(R2=0.87, 0.99, 0.98, respectivamente). Ambas partes del estudio mostraron límites de concordancia satisfactoria con valor medio de la diferencia entre los métodos cercano a 1 en el t30 y tR del TRVP. Conclusión: los métodos dependientes de la medición de la RT son efectivos y confiables para estimar la PMAP. El método de Chemla es útil en el TRVP.

ABSTRACTS: Background: Mean Pulmonary arterial pressure (PMAP)is an indispensable hemodynamic variable for the diagnosis, classification and prognosis of Pulmonary Hypertension (PH). Its quantification is performed invasively by cardiac catheterization and non-invasively by Doppler echocardiography. Masuyama proposed its measurement by the transvalvular diastolic pulmonary gradient derived from the initial maximum velocity of pulmonary regurgitation(ΔPRi2) corresponding closely to the invasive measurement. Objectives: to compare 3 known echocardiographic methods to estimate MPAP and demonstrate the usefulness of the Chemla method in the Pulmonary Vascular Reactivity Test (PVRT). Methods: prospective, observational, double-blind study divided into two stages. A) 30 patients underwent diagnostic Doppler echocardiography. Tricuspid regurgitation (TR) and pulmonary acceleration time (PAT) were measured to derive the equations: 1) 0.61xSPAP + 1.95 (Chemla) 2) Gradient mean pressure TR (ΔPmTR) + RAP (right atrium pressure) (Aduen).3) 79-0.45xPAT o 90-0.60xPAT depending on the value of PAT. B) 10 patients enrolled to PVRT comparing the echocardiographic measurement (Chemla) with right catheterization. Results: in the first part of the study a high correlation between the 3 equations was found : ChemlaAduen, R2 = 0.91; Chemla-Kitabatake, R2=0.87; Aduen-Kitabatake, R2=0.91. In the second part comparing the MPAP-Chemla and RHC we obtained a high correlation in time 0, 30 min and recovery: (R2=0.87,0,99,0.98, respectively). Both parts of the study showed satisfactory limits concordance with mean value of the difference between the methods close to 1 in the t30 and tR of the TRVP. Conclusion: the methods dependent on the measurement of the TR are effective and reliable for estimating MPAP. The Chemla method is useful in the PVRT.

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BACKGROUND: Pulmonary arterial hypertension (PAH) is a fatal complication in patients with connective tissue disease (CTD). OBJECTIVE: The objective of the study was to study the prognostic value of the acute pulmonary vasoreactivity test with inhaled iloprost and its association with clinical deterioration in a tertiary care academic medical center. METHODS: We conducted a prospective study of patients with CTD and the diagnosis of PAH established by right heart catheterization. Patients were classified into classic responders, partial responders, and non-responders. The association of the pulmonary response and clinical deterioration was analyzed. RESULTS: We enrolled 25 patients (mean age of 47 ± 13.4 years); 88% were female. The most frequent rheumatologic diagnosis was systemic lupus erythematosus, in 16 (64%) patients. Seventy-two percent of patients were classified as non-responders, and 28% were partial responders. Patients with a partial response had lower right atrial pressure values (5.1 ± 3.1 vs. 8.5 ± 3.2, p = 0.01) and greater systolic pulmonary arterial pressure (87.6 ± 8.1 vs. 72.4 ± 16.2, p = 0.02), compared with non-responders. Non-responders had a tendency for a shorter time to clinical deterioration than partial responders (17.8 vs. 41.1 months, p = 0.052). CONCLUSIONS: Patients with a partial response to the acute pulmonary vasodilator test with inhaled iloprost had a longer clinical deterioration-free period than non-responders.

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A hipertensão pulmonar é um estado hemodinâmico e fisiopatológico que pode ser encontrado em diversas condições clínicas e quando presente, está associada ao aumento da morbimortalidade. Somados à etiologia diversa estão os achados clínicos inespecíficos. Esses dois aspectos contribuem para o atraso no diagnóstico e, por conseguinte, no início do tratamento. Daí a importância da sistematização da avaliação diagnóstica aliada à suspeição clínica na investigação de dispneia, sobretudo nas populações de risco, visando o diagnóstico etiológico correto e a instituição de medidas terapêuticas adequadas. O seguimento desses pacientes deverá ser feito também de forma sistemática, associado a ferramentas complementares que permitam a avaliação adequada da gravidade da doença...

Pulmonary hypertension is a hemodynamic and pathophysiological state that can be find in various clinical conditions and is associated with increased morbidity and mortality. As symptoms are nonspecific and the condition is uncommon, usually the diagnosis is delayed and, consequently, the start of treatment.Hence, the importance of systematization of diagnostic evaluation combined with clinical suspicion in dyspnea research, especially in at-risk populations, in order to achieve the correct etiologic diagnosis and institution of appropriate therapeutic measures. The follow-up of these patients also should be do systematically, together with complementary tools that allow the proper assessment of disease severity...

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La hipertensión pulmonar constituye una de las enfermedades con creciente prevalencia en la actualidad, con pronóstico ominoso y deterioro en la calidad de vida de los pacientes. El fracaso de la función del ventrículo derecho, consecuencia del remodelado en el lecho vascular pulmonar, que provoca un estado hemodinámico caracterizado por elevación sostenida de la presión en el circuito arterial pulmonar, es el resultado final del aumento de la poscarga. En las últimas décadas, los avances en el conocimiento de esta enfermedad, así como las nuevas investigaciones, han logrado brindar información para llegar al diagnóstico precoz y el tratamiento más adecuado. Ambos constituyen la base para lograr un cambio en la evolución de esta enfermedad, mejorando la calidad de vida y supervivencia de los pacientes. Si bien, es importante el conocimiento por parte de los equipos de salud, la derivación a centros especializados en el manejo de estos pacientes con médicos capacitados contribuirá significativamente a cambiar el curso evolutivo natural de esta enfermedad. La presente revisión tiene como objetivo brindar información actualizada, aportando datos que permitan facilitar la detección precoz, el diagnóstico y el tratamiento de estos pacientes, permitiendo una correcta atención o, en su defecto, realizar la derivación a centros especializados de referencia.

Pulmonary hypertension is a disease with currently increasing relevance, poor prognosis and impaired quality of life of patients. The right heart failure is the end result of the remodeling in pulmonary vascular tree causing a hemodynamic state with sustained elevation of pressure in pulmonary arteries and the subsequent afterload increase. In recent decades, advances in our understanding of this disease, as well as new research has been able to provide information to make an early diagnosis and the most appropriate treatment. Both are the basis for change in the evolution of the disease, improving patient's quality of life and survival. It's also important to change the natural history of the disease, the knowledge of health team and the timely consultation with clinics and physicians with special trainee in management of these patients. This review aims to provide updated information, providing data to facilitate the early detection, diagnosis and treatment of these patients allowing a proper attention or, alternatively, make referrals to specialized referral centers.

A hipertensão pulmonar é uma das doenças com maior relevância no momento, com prognóstico desfavorável nos pacientes e deterioração da qualidade de vida. A falha do ventrículo direito, resultado da remodelação do leito vascular pulmonar causando estado hemodinâmico com elevação sustentada da pressão nas artérias pulmonares e ao aumento da pós-carga é o resultado final. Nos últimos tempos, avanços no conhecimento desta doença bem como a investigação têm fornecido informações para obter o diagnóstico precoce e o tratamento mais adequado. Ambos são a base para as alterações sobre a evolução desta doença, melhorando a qualidade de vida e sobrevida. Embora seja importante o conhecimento das equipes de saúde, ir à clínica e os médicos com estagiário especial na gerência destes pacientes é uma contribuição significativa para alterar a história natural dessa doença. Esta revisão tem como objetivo fornecer informações atualizadas, fornecendo dados para facilitar a detecção precoce, diagnóstico e tratamento destes pacientes permitindo uma atenção adequada ou, em alternativa, fazer encaminhamentos para centros de referência especializados.

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There has been significant progress in our understanding of the pathobiology, epidemiology and prognosis of pulmonary vascular disease and, over the past few years, there has been an explosion of clinical therapeutic trials for pulmonary arterial hypertension (PAH). The increasing number of different conditions now associated with PAH and the appearance of new diagnostic techniques have led to a need for a systematic diagnostic approaches and a new disease classification, which has resulted in notable improvements in the quality and efficacy of clinical care. We appreciate traditional resting right heart catheterization techniques (which still remain the gold standard for diagnosing PAH and managing patients on therapy) and look forward to novel invasive techniques (e.g. intravascular ultrasound) that add greatly to our understanding of right ventricle and pulmonary circulation, and for the interpretation of data from clinical trials as well.

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El diagnóstico final de hipertensión pulmonar se realizamediante cateterismo cardíaco derecho. Existe hipertensiónpulmonar cuando la presión media en la arteria pulmonarsupera los 25 mmHg en situación basal. Una adecuadaexploración hemodinámica permite encuadrar la hipertensiónpulmonar en algunos de los cinco grupos de la clasificaciónclínica actual. Se dan las recomendaciones para los diferentesespecialistas de cuándo efectuar el cateterismo derecho, quéparámetros se deben de registrar y como realizar e interpretaruna prueba aguda de vasorreactividad en la hipertensiónarterial pulmonar.

The final diagnosis of pulmonary hypertension is made byright heart catheterization. Pulmonary hypertension is presentwhen the average pressure in the pulmonary artery exceeds 25mm Hg at baseline. Proper hemodynamic assessment allowsclassification of pulmonary hypertension into one of five clinicalcategories. Recommendations are provided for specialists, whento conduct right heart catheterization, which parameters mustbe recorded and how to perform and interpret a test for acutevasoreactivity in pulmonary arterial hypertensio.

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